Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 7 2 2018 04 01 Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis 45 50 10.15171/jnp.2018.13 EN Jalal Etemadi Taraneh Majidi Roza Motavalli Mortaza Bonyadi Sepideh Zununi Vahed Behzad Zaker Mohammadreza Ardalan Journal Article 10.15171/jnp.2018.13 2017 08 15 2017 09 21 Background: Familial Mediterranean Fever (FMF) is the most common inherited autoinflammatory disease. Kidney involvement in FMF is usually attributed to secondary amyloidosis. Non-amyloid glomerular involvement has also been reported. Objectives: We suppose that heterozygous mutation of Mediterranean fever (MEFV) gene could be the underlying cause in some cases of mesangial proliferative glomerulonephritis (MePGN) in FMF endemic area. Materials and Methods: This prospective study was done between 2013 and 2015 in NorthWest of Iran among the Azari-Turkish population. A panel of MEFV gene including M680I, R761H, M694V, R408Q, E148Q, A744S, F479L, P369S, V726A, M694I, and E167D were studied in a group of patients with idiopathic MePGN. Clinical characteristics and therapeutic responses were compared between those with and without a mutation. A total of 39 idiopathic MePGN patients and 156 healthy subjects were studied. Results: Heterozygote mutations of MEFV gene were detected in 11/39 (28.2%) of MePGN patients and 46/156 (17.3%) of controls. Clinical response regarding 24 hours urine protein excretion was significant in mutation-negative patients after 6 months of follow-up. Conclusions: This study shows a possible underlying role of heterozygous MEFV gene mutation in the clinical course of some case of idiopathic MePGN, particularly in FMF endemic population.